Treatment of Central Adrenal Insufficiency

What is central adrenal insufficiency?

Central adrenal insufficiency is a condition where the adrenal glands do not make enough of a hormone called cortisol.

Cortisol helps the body respond to stress by regulating:

• Blood pressure
• Blood sugar (glucose)
• Immune system function
• Metabolism

In central adrenal insufficiency, the term “central” refers to the cause of the problem being in the brain’s pituitary gland, rather than in the adrenal glands. The pituitary gland is a small, pea-sized organ located at the base of the brain. It releases a hormone called ACTH (adrenocorticotropic hormone). This hormone tells the adrenal glands to make cortisol.

If the pituitary gland does not work properly, it might not send enough ACTH. Then the adrenal glands will not make enough cortisol. This leads to central adrenal insufficiency.

Hydrocortisone for central adrenal insufficiency treatment

The main treatment for central adrenal insufficiency is to replace the missing cortisol with a medicine called hydrocortisone (Cortef^®, Solu-Cortef^®).

Your child will need to take hydrocortisone for the rest of their life. If your child suddenly stops taking hydrocortisone, it can lead to an adrenal crisis. This is a life-threatening emergency where the blood pressure drops very low and may require an emergency dose of cortisol.

Your child needs to take the right amount of hydrocortisone. This amount can change over time.

There are 3 different hydrocortisone dosing procedures, depending on what your child needs:

• Maintenance doses
• Stress doses
• Emergency stress doses

Hydrocortisone is available in these forms:

• 5 mg, 10 mg, and 20 mg white tablets taken by mouth
• An oral liquid to be taken by mouth
• An injection (shot) given into a muscle

Your child can take the medicine with or without food. Always follow the instructions given by your care team.

Maintenance doses of hydrocortisone

Give maintenance doses of hydrocortisone when your child is otherwise healthy. The medicine is given 2–3 times per day. It replaces the cortisol that your child’s body would normally make. These are low doses.

Give the prescribed strength of tablet(s) of the hydrocortisone maintenance dose as follows:

Stress doses of hydrocortisone

Stress doses are higher and mimic the body’s response to stressors, such as illness or injury. The body makes more cortisol during times of stress. The stress dose is usually about 3 times more than the maintenance dose. But always follow your care team’s instructions.

Give stress doses at the first signs or symptoms of an illness or injury. These include:

• Fever:
  • Mouth (oral) temperature of 101°F (38.3°C) or greater
  • Underarm temperature of 101.6°F (38.7°C) or greater
  • Rectal temperature of 100.4°F (38°C) or greater
• Illness such as strep throat, ear infection, bronchitis, pneumonia
• A possible viral illness, such as cold symptoms or runny nose
• Vomiting and diarrhea
• Injuries such as sprains or strains

Give the prescribed stress dose of hydrocortisone every 8 hours.

Stress doses of hydrocortisone can hide a fever. After having a fever, your child needs a medical exam to check for an infection and get treatment. Do not give stress doses for more than 3 days without seeing your health care provider.

Emergency stress doses of hydrocortisone

Always keep an emergency dose of hydrocortisone with your child. In patients with adrenal insufficiency, an adrenal crisis can be a life-threatening emergency. Your child may be too sick to take medicine by mouth.

Symptoms of an adrenal crisis include:

• Low blood pressure
• Low blood sugar (known as hypoglycemia)
• Feeling faint or passing out (unconscious)

If your child has these symptoms, give an emergency stress dose by injection (shot) and get medical help right away.

After giving your child an emergency stress dose, call 911 or go to the nearest emergency room.

Always follow the instructions given by your care team.

How to plan for medical procedures

For any medical procedure, especially those using sedation or general anesthesia, remind your health care provider that your child takes hydrocortisone. Your child may need stress dosing before, during, or after the procedure.

Have your health care provider contact the on-call St. Jude endocrinologist to discuss whether your child needs stress dosing and determine the correct dose.

Tips for families

• Be sure your child takes their hydrocortisone exactly as directed by your care team. Do not skip doses, and do not stop taking the medicine.
• Have your child wear a medical alert bracelet or necklace stating that they have adrenal insufficiency and need steroids for an emergency.
• Prepare a kit with medical supplies that you can use in an emergency. See Emergency Stress Dose for Adrenal Insufficiency for how to make this kit.

When to contact your care team

Contact your care team if your child:

• Is unusually tired or weak
• Has nausea or vomiting that does not go away
• Is dizzy or faints
• Has a fever or an infection
• Feels extremely stressed

These can be signs that your child’s cortisol levels are too low and that they might need a change in medicine.

Questions to ask your care team

• What is my child’s hydrocortisone dose?
• How often will their dose need to be changed?
• What should I do if my child is sick or under a lot of stress?
• How can my child prepare for surgery or other medical procedures?
• What side effects of hydrocortisone should I watch for?
• What should I do in case of an emergency?

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